Nephrotic Syndrome
Protein leaks drive swelling — keep relapses on your radar.
What it is
A kidney disorder where damaged glomeruli leak large amounts of protein into the urine, triggering swelling and other complications.
Prevalence
Fewer than 5 in 100,000 children worldwide are diagnosed each year (approximately 4.7 cases per 100,000).
Symptoms to watch
Puffy eyes and facial swelling, leg and ankle edema, rapid weight gain from fluid, foamy urine, elevated blood pressure, fever or clot-related pain.
Treatment options
ACE inhibitors/ARBs and blood-pressure control, diuretics, cholesterol-lowering agents, steroids and other immunosuppressants, antibiotics, low-sodium diet, vitamin D, blood thinners, and research trials.
Acute Kidney Injury (AKI)
A sudden decline in kidney function — common in hospital settings.
What it is
A rapid loss of kidney function over hours to days, ranging from mild impairment to complete failure.
Prevalence
26% of hospitalized children, 30% of critically ill neonates and children, 30–50% after cardiac surgery, 34% on nephrotoxic medications.
Symptoms to watch
Reduced urine output, swelling, fatigue, shortness of breath, mood changes, high blood pressure, decreased appetite, confusion.
Treatment options
Optimized fluids, medication adjustments, dialysis support when needed, and intensive monitoring.
Glomerulonephritis
Inflammation of kidney filters from immune or infection triggers.
What it is
Inflammation of glomeruli caused by infections or immune overreaction, damaging the filters that remove waste from blood.
Prevalence
APSGN affects approximately 1 in 10,000 kids; primary forms approximately 0.1 per 100,000 annually; a leading cause of CKD in teens.
Symptoms to watch
Blood or foam in urine, swelling of face/limbs/abdomen, high blood pressure, joint or abdominal pain, fatigue, fever, rash, appetite loss.
Treatment options
Blood-pressure meds, diuretics, steroids or immunosuppressives, phosphate binders, antibiotics, dietary salt/protein control, dialysis, or transplant.
Henoch-Schönlein Purpura Nephritis (HSPN)
IgA vasculitis with kidney involvement — watch rash and urine changes.
What it is
Kidney inflammation tied to Henoch-Schönlein purpura, where small vessels become inflamed and leak IgA complexes.
Prevalence
HSP affects 8–20 per 100,000 kids; 5–30% develop nephritis with prognosis tied to kidney involvement.
Symptoms to watch
Blood or protein in urine, microscopic hematuria, joint swelling, abdominal pain, rash, GI bleeding, scrotal swelling.
Treatment options
Supportive care including rest, fluids, and pain relief; corticosteroids; blood-pressure meds; rituximab for chronic cases; tonsillectomy when infection triggers recur.
Minimal Change Disease (MCD)
Leading cause of nephrotic syndrome with microscopic glomerular injury.
What it is
Glomerular damage so subtle it requires electron microscopy, yet drives nephrotic syndrome in children.
Prevalence
10–50 cases per 100,000 children; 2–7 new cases per 100,000 kids each year.
Symptoms to watch
Sudden swelling of face, abdomen, hands, feet; foamy urine; weight gain; high blood pressure; high cholesterol; clot risk.
Treatment options
Corticosteroids first-line, ACE inhibitors, diuretics, sodium restriction, regular exercise, scheduled follow-ups.
Wilms Tumor
Pediatric kidney cancer — detect abdominal masses early.
What it is
A rare malignant kidney tumor in children that grows quickly but responds to coordinated therapy.
Prevalence
Affects roughly 1 in 10,000 children worldwide.
Symptoms to watch
Abdominal lump, blood in urine, fever, nausea, appetite loss, constipation, shortness of breath, high blood pressure.
Treatment options
Surgical removal plus chemotherapy, radiation, and biologic therapies tailored to staging.
IgA Nephropathy (Berger's Disease)
Immune deposits inflame glomeruli — track blood pressure and urine.
What it is
Excess IgA antibodies build up in kidneys, causing inflammation and progressive damage.
Prevalence
Annual incidence in U.S. children is about 0.5 cases per 100,000.
Symptoms to watch
Blood in urine, swelling, flank pain, proteinuria, rashes, itchy skin.
Treatment options
ACE inhibitors/ARBs, corticosteroids and immunosuppressants, diuretics, statins, omega-3 supplements, diet adjustments.
Alport Syndrome
Inherited kidney disease linked with hearing and vision changes.
What it is
A genetic disorder damaging kidney basement membranes with accompanying hearing and eye changes.
Prevalence
Estimated at roughly 1 in 50,000 live births.
Symptoms to watch
Persistent hematuria, progressive kidney decline, hearing loss, eye abnormalities.
Treatment options
ACE inhibitors/ARBs and SGLT2 inhibitors, healthy lifestyle, hearing aids, ophthalmologic care, transplant for end-stage disease, genetic counselling.
Gitelman Syndrome
Inherited electrolyte imbalance with salt cravings and muscle cramps.
What it is
A rare genetic kidney disorder that disrupts electrolyte handling, leading to chronically low potassium and magnesium.
Prevalence
Approximately 1 in 40,000 people worldwide; symptoms typically appear in late childhood or adolescence.
Symptoms to watch
Muscle cramps or spasms, fatigue, paresthesias, salt cravings, low blood pressure, excessive thirst and urination, GI upset, joint calcifications.
Treatment options
Dietary sodium and potassium, magnesium supplementation, potassium-sparing diuretics, pain relief, regular monitoring, cardiac evaluation.
Nephronophthisis
Genetic tubulointerstitial scarring leading to childhood kidney failure.
What it is
A ciliopathy that causes interstitial fibrosis and cyst formation, ultimately progressing to kidney failure.
Prevalence
Estimated 1 in 50,000 newborns in Canada, 1 in 100,000 in Finland, and 1 in 922,000 in the United States.
Symptoms to watch
Excessive thirst and urination, fatigue, weakness, anemia, development of kidney cysts.
Treatment options
Close monitoring, blood-pressure and anemia management, addressing structural anomalies, and dialysis or transplant when function declines.
Medullary Cystic Kidney Disease (MCKD)
Autosomal dominant tubulointerstitial kidney disease progressing to ESRD.
What it is
A genetic disorder that forms cysts in the medulla, causes salt wasting, and leads to gradual kidney failure.
Prevalence
Rare; accounts for 10–25% of pediatric end-stage renal disease cases.
Symptoms to watch
Easy bruising, fatigue, itching, muscle twitching, gout, excessive urination, salt craving, weight loss, seizures.
Treatment options
Hydration and salt supplements, medication and diet changes to slow decline, dialysis, and transplant for advanced disease.
Tuberous Sclerosis Complex — Renal (TSC)
Multisystem genetic disorder with angiomyolipomas and cysts in kidneys.
What it is
A genetic condition causing benign tumors and cysts across organs, with kidneys commonly affected.
Prevalence
Kidney involvement occurs in 70–80% of children with TSC, most often during childhood or adolescence.
Symptoms to watch
Angiomyolipomas, renal cysts, occasional bleeding, anemia, rare renal cell carcinoma, high blood pressure.
Treatment options
Embolization for bleeding AMLs, surgery when necessary, everolimus to slow tumor growth, blood-pressure control, dialysis, and transplant in severe cases.
Hemolytic Uremic Syndrome (HUS)
Microangiopathy linking infection, anemia, and acute kidney injury.
What it is
A condition marked by hemolytic anemia, thrombocytopenia, and acute kidney injury, often following GI illness.
Prevalence
0.6–0.8 cases per 100,000 children annually, rising to 1.9–2.9 per 100,000 in children under five.
Symptoms to watch
Bloody diarrhea, fever, vomiting, lethargy, irritability, decreased urine output, pale skin, neurologic changes.
Treatment options
Fluids and electrolytes, transfusions, dialysis, medications including eculizumab, intensive care, nutrition support, transplant if kidneys fail.
Fabry Disease
Lysosomal storage disorder with multi-organ kidney involvement.
What it is
An X-linked deficiency of alpha-galactosidase A leading to Gb3 accumulation and progressive organ damage.
Prevalence
Rare and frequently underdiagnosed; pediatric prevalence not well defined.
Symptoms to watch
Angiokeratomas, decreased sweating, neuropathic pain, GI issues, cloudy vision, hearing loss or tinnitus.
Treatment options
Enzyme replacement therapy, oral chaperone therapy for specific mutations, diet support, and symptom-based management.
SPLIS (Sphingosine Phosphate Lyase Insufficiency Syndrome)
Rare SGPL1-related disorder affecting kidneys, adrenal glands, and the nervous system.
What it is
A rare inherited disorder caused by SGPL1 mutations, often linked to steroid-resistant nephrotic syndrome, adrenal insufficiency, and neurologic complications.
Symptoms to watch
Kidney disease, seizures, developmental delays, adrenal issues, immune and gastrointestinal concerns, and skin abnormalities.
How diagnosis is confirmed
Diagnosis is confirmed when symptoms are present and genetic testing identifies two faulty SGPL1 gene copies.
Treatment approach
No cure yet. Care focuses on early diagnosis, symptom management, hormone therapy, dialysis, immune monitoring, and nutritional support. Research into gene therapy and vitamin B6 as a cofactor is ongoing.
